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Vampires, giants, zombies: how rare diseases gave rise to world legends

Why do people believe in vampires, zombies, and curses?
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Before the advent of the microscope, genetics, and the theory of microbes, mankind had only one tool to explain the inexplicable—imagination. Monsters weren't born from the void. Behind each of them — vampire, werewolf, zombie, giant — there is a real person with a real diagnosis. History knows dozens of cases when an unnamed disease became a legend with a thousand-year biography. How rare diseases became legends and where mythology ends and genetics begins — in the material of Izvestia.

Vampire Disease: why they were afraid of the sun and garlic

Pale skin, intolerance to sunlight, receding gums, exposing teeth and an acute reaction to garlic: if you describe the symptoms of porphyria to a person who is not familiar with medicine, he will most likely call it a portrait of a classic vampire. It was this connection that biochemist David Dolphin first formulated publicly in 1985 in a speech before the American Association for the Advancement of Science.

Izvestia reference

Porphyria is a group of rare genetic disorders in which the synthesis of heme, an iron—containing component of hemoglobin, is disrupted. As a result, porphyrins accumulate in the tissues, substances that, upon contact with ultraviolet light, trigger destructive reactions. The skin becomes inflamed, blistered and scarred, and darkens. In some forms of the disease, the nervous system is affected: hallucinations, disorientation, seizures.

All types of porphyria are rare, and the disease remains incurable to this day. Treatment is limited to stopping seizures and strictly avoiding provoking factors. Among them is garlic: it is capable of launching acute attacks in carriers of certain forms of the disease.

In the chronic course of the disease, the tissues around the teeth degrade, the teeth become exposed and visually resemble canines. As Michael Hefferon, a professor at Queens University, points out, it was the combination of these symptoms observed in Balkan villages, where porphyria was historically more common due to isolated populations, that became the source material for vampire folklore.

The two most frequently named historical bearers of porphyria are Wallachian Prince Vlad III (the prototype of Dracula in Bram Stoker's novel) and British King George III, whose "madness" is documented by court chronicles. As noted in the UPMC Research Blog, Vlad III avoided daylight, and George III's behavior during seizures was described as disorientation and hallucinations — symptoms characteristic of acute forms of porphyria. Both diagnoses remain retrospective and debatable.

Izvestia reference

Vampire folklore developed in the Balkan region — in the territories of modern Serbia, Romania, and Bulgaria — around the 17th and 18th centuries and was recorded in the Austrian military reports of the 1720s. The first literary vampire did not appear in Stoker: in 1819, the Anglo-Italian physician John Polidori published the novel "The Vampire", anticipating "Dracula" by eight decades. Polidori was Lord Byron's personal physician.

Strasbourg, 1518: 400 people danced to death

In July 1518, a resident of Strasbourg — she appears in the chronicles as Frau Trotta — went outside and began dancing. Without music, without stopping, without responding either to her husband's requests or to the heat. She danced for several days in a row. Then others joined her. By August, according to chroniclers, uncontrolled movements had engulfed from several dozen to four hundred townspeople. Some sources report 15 deaths per day from exhaustion, stroke and heart attack, although the Strasbourg city archives did not record specific figures.

Eventually, public dancing was banned and the most seriously injured were sent to the sanctuary of St. Vitus, a Catholic martyr who was prayed for deliverance from the "dancing disease." Modern science considers two main explanations. The first is ergotism: poisoning by alkaloids of the fungus Claviceps purpurea, which parasitizes rye. Its toxins cause seizures, hallucinations, and necrosis of the extremities. The second, and by far the most accepted in the scientific community, is mass psychogenic illness (BCH).

As The Public Domain Review writes in one of the most detailed studies of the event, "the suffering of 1518 took the form of a dance because the townspeople believed that it could manifest itself in this way." Strasbourg was well aware of the precedents: all previous "dancing epidemics" between 1374 and 1518 occurred within a radius of several hundred kilometers from it.

Izvestia reference

The "dancing plague" is just one of the forms of choreomania that swept through medieval Europe. The first documented wave dates back to 1374 and covered the Rhineland. The Dance of St. Vitus, the Dance of Death (Danse Macabre) and tarantism are considered cultural and medical phenomena. The Strasbourg epidemic of 1518 was the last recorded in Western Europe.

Anthropologist Wade Davis and the Real Zombie: The Story of Clervius Narcissus

The Haitian zombie is not a Hollywood ghoul with a craving for brains. In voodoo culture, a zombie is a living person stripped of his will and personality and turned into a submissive tool. Haitians have historically been afraid not of zombies, but of becoming zombies.

In 1962, a man was admitted to the Duchene Hospital in Haiti complaining of fever, weakness, and aches. He was examined by two doctors, pronounced dead and buried. His name was Clervius Narcissus. In 1980, he appeared to his sister at the village market, alive, in full memory, with a scar that matched the nail from the coffin lid. According to him, he was taken out of the grave, drugged and used for two years as a labor force on a sugar plantation.

This case attracted the attention of Harvard ethnobotanist Wade Davis, who conducted three expeditions to Haiti in 1982-1983 and collected samples of "zombie powder" from several practicing voodoo bokors. His research formed the basis of the book The Serpent and the Rainbow (1985) and an article in Harper's Magazine (1984). According to Harvard Magazine, Davis found tetrodotoxin in each of the powder samples obtained.

Izvestia reference

Tetrodotoxin is one of the strongest non—protein neurotoxins known to science. It is found in puffer fish (pufferfish), some salamanders and octopuses. The mechanism of its action is simple and cruel: the molecule blocks the sodium channels of neurons, preventing the nerve impulse from reaching the muscles. At a dosage close to fatal, the victim's metabolism slows down to a state almost indistinguishable from death: the pulse is almost undetectable, breathing becomes imperceptible.

This is exactly the dose that Davis hypothesized the future "zombie" would receive. He was buried alive, dug up, and then given Datura stramonium (datura), a plant with a pronounced psychoactive effect that causes disorientation and amnesia, and psychological suppression was reinforced through the cultural context of voodoo.

The scientific community has ambiguously accepted the hypothesis. A laboratory analysis published on PubMed confirmed the presence of tetrodotoxin in Haitian powder samples. Critics, however, pointed out that the concentrations of the toxin in the studied samples were too low to reliably reproduce the described effect and that Davis' methodology for collecting the material raised questions. Davis himself admitted that "the threshold between the dose that imitates death and the dose that causes it is catastrophically narrow."

Marfan Syndrome: Why "giants" scared medieval people

Marfan syndrome occurs in about one in 5-10 thousand people and is caused by a mutation of the FBN1 gene on chromosome 15, responsible for the production of fibrillin, a protein that forms the framework of connective tissue. Its carriers are usually disproportionately tall, have long, thin limbs, spider-like fingers, a pronounced scoliotic arch, and severe aortic vulnerability. It is the rupture of the aorta that is the main cause of early death in Marfan syndrome; before the widespread use of cardiac surgery, most carriers did not live to be 40 years old.

Izvestia reference

The mythology of giants is universal: Greek giants, Scandinavian jotuns, the biblical Goliath, the Irish Fomor. In all traditions, a giant is a being on the border of the human and the inhuman: excessively strong, excessively fragile, doomed. Medical historians believe that this is not a coincidence: in every culture, people with growth disorders were periodically born — Marfan syndrome, acromegaly, gigantism of pituitary origin — and each time their unusual appearance and early death received a mythological form long before the medical one.

The alleged historical bearers include Abraham Lincoln, Hans Christian Andersen and Julius Caesar. The discussion around Lincoln is documented in the most detail: in 1964, California doctor Harold Schwartz published an article in JAMA arguing for the diagnosis, based on the fact that one of the president's distant relatives, who traced his ancestry to the same ancestor, Mordecai Lincoln II, was diagnosed with Marfan syndrome. 193 centimeters tall, disproportionately long arms and fingers, elongated face, visual impairments — all this coincides with the classic picture.

Nevertheless, there is still no molecular genetic confirmation of Lincoln's diagnosis: requests for analysis of preserved biological samples have been repeatedly reviewed and rejected on ethical grounds.

As for Andersen, the Marfan Trust draws attention to the fact that the future author of The Ugly Duckling has been ridiculed since childhood precisely because of his height and physique, and the Ugly Duckling itself is traditionally read as an autobiographical metaphor for a man whose "strangeness" was perceived as a flaw during his lifetime.

"Royal Illness": how hemophilia became a generic curse

Hemophilia B appeared in the British royal family suddenly — without any predecessors on the paternal or maternal side. According to Science estimates, the probability of such a spontaneous mutation is one case per 50 thousand. The carrier turned out to be Queen Victoria, who was outwardly healthy, but passed on the defective gene to her sons, who developed the disease, and to her daughters, who spread it further through dynastic marriages.

Izvestia reference

Hemophilia B is a hereditary blood clotting disorder in which the body does not produce enough factor IX. Even a small cut or bruise can cause unstoppable bleeding, whether external or internal. Before the invention of clotting factor-based drugs in the 1960s, the disease was virtually untreatable. Today, carriers of hemophilia B lead almost normal lives thanks to regular infusions of recombinant factor IX.

The consequences manifested themselves in several generations at once. Victoria's son Prince Leopold died at the age of 31 from a hemorrhage after a minor fall. Grandson Friedrich — at the age of two. Two more grandchildren did not live to be forty. The disease spread to the thrones of Great Britain, Germany, Russia and Spain as the female heiresses entered into dynastic marriages.

The Russian branch turned out to be the most vulnerable. As historian Helen Rappaport clarified in an interview with the National Bleeding Disorders Foundation (NBDF), Tsarevich Alexei, born in 1904, received a severe form of the disease: bruises from the slightest blows, bleeding in the joints, weeks in bed, agonizing pain. No medical means of that time helped.

"If Alexey hadn't been born a hemophiliac, there would have been no need for secrecy. The throne would not have been in danger, because Alexandra would not have clung so tightly to Rasputin, who was hated and demonized. In many ways, Alexei's hemophilia triggered the mechanism of the fall of the monarchy, because Alexandra was so hated. And when she made Rasputin a friend and adviser, the outcome was a foregone conclusion: too many in Russia hated them both and considered them spies," said Rappaport.

Today, the disease is controlled: patients are regularly injected with recombinant factor IX, a synthetically produced protein that replenishes what the blood lacks for normal clotting. Modern drugs allow for infusions once every one to two weeks. The gene therapy approved in a number of countries in 2022-2023 gives hope that this need will disappear in the near future.

The End of the Age of Curses: Scientific evidence

For most of human history, the disease has been attributed to three causes: the wrath of the gods, the "evil air" and the imbalance of humors. The wrath of the gods presupposed the search for the culprit — witches, heretics or lepers: in every medieval epidemic there was someone who was responsible for the "spoilage".

The miasma theory, according to which diseases spread through polluted air, led to the appearance of the characteristic masks of plague doctors: their beaks were filled with aromatic herbs designed to "purify" the air. Humoral theory, dating back to Hippocrates and Galen, linked mental disorders with an excess of "black bile", which is why people with hallucinations or seizures were often considered possessed.

The first to suggest the living nature of the infection was the Jesuit scientist Athanasius Kircher: in 1658, he described microscopic "worms" in the blood of plague victims and proposed measures such as isolation, burning infected clothing and using protective masks. However, his ideas were not recognized.

The turning point occurred only two centuries later: in the 1860s, Louis Pasteur refuted the theory of spontaneous generation and proved the role of microorganisms in the processes of fermentation and putrefaction. In 1876-1883, Robert Koch identified the causative agents of anthrax, tuberculosis and cholera and formulated criteria for a causal relationship between a microbe and a disease - Koch's postulates. The development of microbial theory has made mystical explanations redundant: porphyria has been explained as a violation of porphyrin metabolism, and hemophilia as an X-linked recessive disease with a developed therapy.

Переведено сервисом «Яндекс Переводчик»

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